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Table 1 Classification of interstitial lung diseases

From: Interstitial lung diseases in the hospitalized patient

Classification

Clinical-radiological-pathological diagnosis

Histopathologic pattern

ILDs of known cause

Environmental or drug related

Depends on underlying disease

Connective tissue disease related

Depends on underlying disease

Hypersensitivity pneumonitis

 

Idiopathic interstitial pneumonias (IIPs)

  

 Major IIPs

  

  Chronic fibrosing IPs

IPF

UIP

Idiopathic NSIP

NSIP

  Smoking-related IPs

DIP

DIP

RB-ILD

Respiratory bronchiolitis

  Acute/subacute IPs

COP

Organizing pneumonia

AIP

Diffuse alveolar damage

 Rare IIPs

Idiopathic LIP

LIP

Idiopathic PPFE

Elastotic fibrosis of pleura and subpleural parenchyma

 Unclassifiable IIPs

  

Granulomatous lung disorders

Sarcoidosis

Non-necrotizing granuloma

Other forms of ILD

LAM

Cysts and proliferation of LAM cells

PLCH

Proliferation of Langerhans cells

Eosinophilic pneumonia

Depends on disease onset

  1. AIP acute interstitial pneumonia, COP cryptogenic organizing pneumonia, DIP desquamative interstitial pneumonia, IP interstitial pneumonia, IPF idiopathic pulmonary fibrosis, LAM lymphagioleiomyomatosis, LIP lymphocytic interstitial pneumonia, NSIP nonspecific interstitial pneumonia, PLCH pulmonary Langerhans cell histiocytosis, PPFE pleuroparenchymalfibroelastosis, RB-ILD respiratory bronchiolitis-associated interstitial lung disease, UIP usual interstitial pneumonia